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Primary myelosarcomas are associated with a high rate of relapse: report on 34 children from the acute myeloid leukaemia–Berlin–Frankfurt–Münster studies
Author(s) -
Reinhardt D.,
Pekrun A.,
Lakomek M.,
Zimmermann M.,
Ritter J.,
Creutzig U.
Publication year - 2000
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1046/j.1365-2141.2000.02290.x
Subject(s) - medicine , myeloid leukaemia , incidence (geometry) , cumulative incidence , bone marrow , myeloid , chemotherapy , log rank test , pediatrics , overall survival , gastroenterology , cohort , physics , optics
Primary myelosarcomas are rare manifestations of acute myeloid leukaemia (AML) that precede bone marrow involvement. Out of 744 children observed during the AML–Berlin–Frankfurt–Münster (BFM) studies 87 and 93, 34 children presented with extramedullar myelosarcomas and no blasts ( n = 21; 2·8%), or a low blast count ( n = 13; 1·7%) in the bone marrow. Owing to the initially mild and variable symptoms, in some children ( n = 12) diagnostic procedures were delayed and treatment intensity was reduced. At 0·65 ± 0·13, the cumulative incidence of relapse was significantly higher than in other AML patients (0·28 ± 0·02). The 5‐year event‐free survival was 0·19 ± 0·08 (compared with 0·48 ± 0·02 in AML‐BFM studies 87/93; P (log rank) < 0·03). Overall, 18 out of 34 patients died from disease (estimated 5 year survival 0·44 ± 0·09 compared with 0·55 ± 0·02 in the AML‐BFM‐studies 87/93; P (log rank) = 0·35, n.s.). An early diagnostic workup is needed in children with unusual skin lesions or tumours, considering myelosarcoma as a primary manifestation of AML. Intensive AML‐specific chemotherapy is recommended soon after diagnosis.